ALU 301 - Ch 11: Neurological Disorders

1. __________ is best described as a loss of muscle function in one or more muscle groups.

2. ________ is incomplete or partial paralysis.
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1. Paralysis is often the result of trauma to the brain or spinal cord, but other major causes include tumors, vascular insult, motor neuron or __________ diseases, inflammation, and infection.

2. Paralysis is often described by the clinical picture presented, the geographic area of the body affected, or the ______ of the nervous system affected (e.g., cerebellar-pontine lesion).
Certain terms are often used when describing paralysis of specific areas of the body:

1. monoplegia/monoparesis: paralysis or paresis of a single limb

2. diplegia: paralysis that's __________, usually affecting lower limbs more than upper limbs. This term is often used when describing the effects of cerebral palsy

3. hemiplegia/hemiparesis: paralysis or paresis affecting one side of the body. The most common cause of hemiparesis is __________ disease, with the upper limb usually more severely affected than the lower limb. Depending on the area of the brain affected, facial muscles can be involved.

4. paraplegia/paraparesis: paralysis or paresis of the lower trunk and legs (usually below a spinal cord lesion). Sensory, as well as motor nerves, are often affected.

5. quadriplegia/tetraplegia and tetraparesis: the most severe form of paralysis or paresis affecting both arms and both legs. It's commonly caused by trauma - usually injury to the high cervical spinal cord region (C4 or higher). There is always ________ and bladder involvement, and respiratory muscles can be compromised depending on the level of injury; assisted ventilation can be required to support respiration.
Besides defining paralysis by the area of the body affected, physiologic characteristics are often noted to be spastic or flaccid.

1. Spastic paralysis is associated with motor nerve damage in the central nervous system (i.e., brain and spinal cord). It's manifested by exaggerated deep tendon reflexes and forceful and prolonged muscular spasms. Over time, limbs can be fixed in bent positions known as ___________.

2. Flaccid paralysis is often associated with cranial or spinal nerve lesions and causes loss of __________ tone.
Key underwriting considerations for proposed insureds with paralysis or neuropathies include:

1. _____ at onset

2. underlying _______

3. whether it's acute, chronic, remitting/recurring, stable, or progressive

4. impact on quality of life, if any (e.g., vocation, avocation, ADLs, psychological)

5. degree to which it affects physiologic functions (e.g., breathing, eating, bowel, and bladder)
1. The conditions are characterized by motor deficits that limit activity, but individuals with CP often have associated musculoskeletal problems, learning disabilities, and __________ problems.

2. CP usually involves one of the previously mentioned forms of spastic or flaccid paralysis or paresis, with varying degrees of ___________ function (ranging from completely normal to profoundly impaired), sensorineural loss (visual and auditory), and seizures.
CP has three major subgroups:

1. Dyskinesia syndromes are often associated with the hemiplegic or diplegic forms of CP. They're characterized by involuntary movements creating abnormal postures and/or facial grimaces. The dyskinesia is described by the movements and can be present singularly or mixed. The movements are:
a. athetoid: slow, smooth writhing of distal muscles
b. chorea: irregular, _________ contractions of single muscles or muscle groups
c. dystonia: __________, patterned twisting of the trunk and limbs

2. __________ cerebral palsy is the result of upper motor neuron disease and characterized by hyperreflexia, spastic hypertonia (velocity-dependent, increased resistance that occurs in response to passive muscle stretch) and retention of primitive reflexes.

3. The ataxic subgroup involves _____ abnormalities.
1. Cerebral US, MRI, electroencephalography, and sensory evoked potentials are used to evaluate the degree of ___________ involvement and support the clinical diagnosis. 2. Treatment is largely aimed at maintaining __________, where possible, and will usually include physiotherapy and a variety of exercises. 3. Orthopedic devices can be used to help with mobilization, and surgery is sometimes necessary to lengthen tendons or realign limbs. ________ therapy can also be useful to improve swallowing and communication skills.1. neurological 2. mobility 3. SpeechThe prognosis of those with cerebral palsy is largely dependent on the severity of the symptoms. Underwriting considerations include: 1. extent and severity of the disability 2. degree of ___________ impairment, if any 3. history of ___________ 4. ability or inability to live independently and perform ADLs 5. independent mobility or need for assistive devices2. intellectual 3. seizuresFavorable features of cerebral palsy include monoplegia or __________ only, no evidence of intellectual impairment, no history of seizures, and ability to live independently, with good motor skills and ability to walk unaided.hemiplegia1. Common causes of spinal cord injury in the US are _______, falls, violence, and sporting injuries. 2. Between males and females, spinal cord injuries are more common in ______ and in those under 30 years of age. 3. True or False: There is usually complete loss of motor and sensory function below the level of the lesion and bowel and bladder involvement are not uncommon.1. motor vehicle accidents 2. males 3. TrueSpinal cord damage is usually associated with injury to the vertebral column, and the area of injury is often defined by which vertebral area is affected (for ex, a lesion between the 5th and 6th cervical vertebrae is a C5-6 lesion). 1. Motor and sensory function, reflexes, and sphincter tone are _________ above the area of the lesion and diminished or absent below. 2. Spinal cord injuries can be complete (no sensory or motor function below the lesion) or __________ (some motor and sensory function remains).1. normal 2. incompleteFor spinal cord injuries, the degree of impairment is measured using the __________ Spinal Cord Injury Association (ASIA) scale.AmericanThe prognosis of those paralyzed as a result of a spinal cord injury is variable and dependent on a number of factors. Favorable features include: 1. One limb only affected, fully ambulatory 2. ___________ function preserved 3. Bowel and bladder function not affected 4. ________ function normal 5. Able to live independently 6. Well adjusted to condition 7. No evidence of associated substance abuse2. Respiratory 4. RenalUnfavorable features of a spinal cord injury include: 1. Multiple limbs affected, wheelchair dependent 2. Artificial ventilation required 3. Bowel and bladder function impaired 4. Impaired renal function 5. Requires assistance for _____ 6. Poor adjustment to condition, perhaps with a history of _________ 7. Evidence of drug use or excess alcohol consumption5. ADLs 6. depression1. For spinal cord injuries, _________ is the initial treatment if stabilization is necessary. 2. Physical and occupational __________ are the key to maximizing the function that remains.1. surgery 2. therapyPeripheral __________ is caused by damage to the peripheral nervous system, which is the communicating network between the central nervous system (the brain and the spinal cord) and the rest of the body.neuropathyPeripheral neuropathy usually affects sensation but can also involve motor function, resulting in a wide range of signs and symptoms including: 1. neuropathic symptoms (e.g, _________, altered sensation, burning pain) 2. muscle _________, altered ankle reflexes 3. organ or gland dysfunction 4. changes on electromyography (EMG) and nerve conduction studies (NCS)1. numbness 2. weaknessMononeuropathies affect a single nerve. Examples are: 1. ______ palsy: facial nerve paralysis often caused by a virus or trauma 2. carpal tunnel syndrome: median nerve inflammation caused by repetitive trauma, producing paresthesias (numbness and tingling) in the thumb, index, and middle fingers 3. _________ outlet syndrome: pain and paresthesia of the arm and hand caused by compression of the brachial nerve 4. mononeuritis multiplex: an inflammatory mononeuropathy that involves nerves in at least _____ areas; it's associated with systemic lupus, vasculitis, and other conditions1. Bell's 3. Thoracic 4. twoUnderwriting information for mononeuropathies, such as Bell's palsy or carpal tunnel syndrome, should include: 1. age of onset 2. extent of recovery 3. any residual _________ 4. any evidence of _________ episodes3. disability 4. recurrent______________ affect multiple nerves and can occur in acute forms, such as Guillain-Barre syndrome, or in chronic forms, such as chronic inflammatory demyelinating polyneuropathy (CIPD). Many have no identifiable etiology.PolyneuropathiesAutonomic neuropathy is a peripheral nerve dysfunction that affects nerves involved in regulating involuntary muscle involved in: 1. maintaining ________ rate 2. _________ pressure 3. _________ motility 4. perspiration 5. pupil dilation 6. glandular function1. heart 2. blood 3. gastricSymptoms of autonomic neuropathy include syncope, inability of the _________ to respond to changing conditions, gastroparesis, urinary retention, erectile dysfunction, decreased _________, and inability of the pupil of the eye to adjust to a change in light.heart, sweating___________ neuropathies most commonly arise as a result of a disease process, although injuries, such as those seen in acute or repetitive trauma, can also lead to neuropathies.PeripheralCommon disease-related causes of peripheral neuropathy include: 1. ____________ disorders (e.g., diabetes, hypothyroidism) 2. generalized vascular disease 3. ____________ diseases (e.g., Charcot-Marie-Tooth, Friedreich's ataxia) 4. toxicity (e.g., alcohol abuse, heavy metal ingestion) 5. connective tissue and chronic inflammatory disorders (e.g., systemic lupus, rheumatoid arthritis) 6. vitamin deficiencies (e.g., B12 deficiency) 7. __________ (e.g., leprosy, HIV) 8. paraneoplastic syndromes1. metabolic 3. hereditary 7. infectionDiagnosis of peripheral neuropathy can be difficult because it presents with a vast number of possible signs and symptoms. 1. An extensive history, clinical examination, and a variety of tests are often necessary to reveal a systemic cause for the neuropathy. Testing is likely to include blood tests (i.e., vitamin levels, thyroid, liver and kidney function tests, and various autoantibodies), __________ conduction studies, CT and MRI scans, and electromyography. 2. A nerve ________ is often necessary.1. nerve 2. biopsy1. The most effective treatment for neuropathy is the _______ of the underlying disease. 2. Gabapentin (Neurontin), duloxetine (Cymbalta), pregabalin (Lyrica), and tricyclic antidepressants are used to reduce pain associated with neuropathies. Analgesics and low dose __________ should be used sparingly for breakthrough pain.1. control 2. narcoticsThe prognosis for motor-sensory neuropathies is dependent on the underlying cause. 1. Most mononeuropathies are considered to be _______ conditions with little adverse effect on mortality. 2. On the other hand, some neuropathies can be symptoms of more severe underlying pathology that have significant associated mortality risks.1. benign1. Diabetic neuropathy is damage to sensory nerve fibers or autonomic nerves due to prolonged periods of __________. 2. Motor function is often __________.1. hyperglycemia 2. sparedDiabetic neuropathy onset is gradual with damage resulting in: 1. loss of ________ sensation and proprioception (i.e., the unconscious ability to sense movement and the body's orientation in space) 2. impaired sensation to _____ and temperature1. vibratory 2. pain1. The most common presentation of diabetic neuropathy is a symmetrical ____________ that initially affects the lower extremities, followed later by the hands, in what is described as a stocking-glove distribution. 2. Typical complaints are usually that of burning sensation, pain, numbness, and tingling. Because of sensory loss, there's an increased risk of trauma, ulceration, and infection, particularly of the _____.1. polyneuropathy 2. feetTesting for diabetic neuropathy includes: 1. general examination of the feet 2. evaluation of _________ 3. ____________ sensation (done with a tuning fork) 4. monofilament testing or pinprick (specific areas of the foot are tested for pressure and sensation)2. reflexes 3. vibratoryIn some cases, diabetic neuropathy affects the __________ nervous system, and these findings include gastric paresis (stomach empties slowly) and GI problems (i.e., bloating, diarrhea, constipation), syncopal symptoms, hypotension, tachycardia, and erectile dysfunction.autonomicThe management of diabetic neuropathy includes: 1. ruling out other causes of peripheral neuropathy 2. control of hyperglycemia 3. education of the individual in protecting the feet and hands from environmental risks 4. periodic ______ examination by the PCP 5. treating the _________ symptoms symptomatically4. foot 5. autonomicTrue or False: Diabetic neuropathy is often an indicator of end organ damage from diabetes.True1. __________ inflammatory demyelinating polyneuropathies include Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). 2. Unlike many neuropathies, these can usually be treated ____________.1. Acquired 2. successfullyGuillain-Barre is an immune-mediated inflammatory condition. 1. Etiology is unknown but onset is often noted after a mild bacterial or viral _________. 2. Progressive weakness usually presents in the legs and progresses (over hours to weeks) to the upper body and arms, with varying degrees of paralysis/paresis. In severe cases, there can be total body _________ with respiratory distress necessitating ventilatory support. 3. Autonomic symptoms occur including cardiac __________ and severe lability in blood pressure. 4. Most will make a full recovery within a few weeks to months, but in a small percentage of cases it can lead to prolonged or permanent disability, and recurrent episodes can be indicative of CNS _________.1. infection 2. paralysis 3. arrhythmias 4. demyelination1. True or False: A spinal tap can provide valuable information in Guillain-Barre. 2. Treatment includes plasmapheresis and intravenous immunoglobulins (IVIG). __________ have no therapeutic effect and may be deleterious. 3. Physiotherapy is often an adjunct to maintain muscle function and mobility.1. True 2. GlucocorticoidsThe underwriting info that should be obtained for cases of Guillain-Barre syndrome includes: 1. confirmation of diagnosis 2. course of disease (i.e., acute, self-limiting episode or chronic, relapsing course) 3. whether a full ________ has been made 4. details of any residual deficit 5. whether there's been more than one distinct ___________3. recovery 5. episodeChronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated disorder. It's the chronic counterpart of Guillain-Barre syndrome. It's also called chronic __________ polyneuropathy.relapsingSigns and symptoms of CIDP include: 1. _________, proximal, and distal motor and/or sensory muscle dysfunction lasting for at least eight weeks 2. diminished tendon ________ 3. alterations in proprioception1. symmetric 2. reflexes1. CIDP is found in young adults. Is it more common in males or females? 2. MRI, nerve biopsy, and spinal taps provide confirmation of __________. 3. Treatment includes glucocorticoids, IVIG, and plasmapheresis. Gapabentin (Neurontin) and tricyclic antidepressants are used for pain; ___________ are used if other treatments are ineffective. 4. True or False: Physical therapy is not essential.1. Males 2. diagnosis 3. immunosuppressives 4. False. PT is essential for maintenance of muscle strength and function.Multifocal motor neuropathy (MMN) is an immune-mediated, demyelinating neuropathy. 1. MMN is characterized by slowly progressive muscle _________, fasciculations, and cramping of muscles. 2. Sensory involvement is minimal or __________. 3. Onset is often ___________, affecting a single peripheral nerve causing wrist drop, foot drop, or grip weakness.1. weakness 2. absent 3. asymmetricMMN is a rare condition. 1. Are males or females more frequently affected? 2. Mean age at onset is around 40 years of age. 3. Asymmetric involvement is observed in over 90% of patients and can persist throughout the course of the illness. What is more common: weakness in the upper limbs or lower limbs?1. Males 3. upper limbsNerve conduction studies are crucial with MMN. 1. Multifocal demyelination confined to the motor nerves is present, with partial motor conduction ________ being the hallmark of the disease. 2. Anti-GMI antibodies are elevated in 50% of those with this condition. There is usually improvement in muscle ________ 3-6 weeks after commencement of treatment, usually with intravenous cyclophosphamides (Cytoxan) or immunoglobulin (IVIG), though other immunosuppressives can be used. 3. Those individuals who receive treatment early are likely to experience little, if any, ________, although there's evidence of very slow progression over many years.1. block 2. strength 3. disabilityHereditary disorders, such as Charcot-Marie-Tooth (CMT), also cause peripheral neuropathies. 1. CMT is a heterogeneous group of genetically-distinct disorders that have a similar clinical presentation. Those with autosomal __________ forms of the disease usually present with weakness, muscle wasting, and sensory loss (predominantly in the lower legs) in the first two decades of life. 2. Those with autosomal ________ forms usually develop symptoms of leg weakness in childhood and are unable to walk by adolescence.1. dominant 2. recessiveCMT is one of the more common of the heritable neurological disorders. True or False: It's found world-wide among people of all races and ethnic groups.True, although certain autosomal recessive mutations may be more prevalent in specific ethnic groupsThere are seven different types of CMT with CMT1 and CMT2 being the more common variants. 1. CMT1 is caused by a mutation in the peripheral myelin protein 22 (PMP 22) gene. The result of the genetic defect is production of myelin that's unstable and spontaneously breaks down, causing _____________ as evidenced by slowing of conduction velocity on nerve conduction studies. 2. CMT2 is primarily an axonal disorder rather than a demyelinating disorder. Type 2 results in peripheral neuropathy through direct _______ death.1. demyelination 2. axonalTreatment of CMT is limited. 1. Daily stretching exercises are useful to prevent ankle and foot contractures. Orthopedic ________ is often necessary. 2. CMT is a slowly progressive disorder that can lead to disability in later life, but most of the affected individuals have a _________ life expectancy.1. surgery 2. normalUnderwriting information for CMT that should be obtained includes: 1. confirmation of diagnosis 2. level of current function 3. degree of ___________progressionDementia is commonly thought of as a disease which mainly affects older people, but it can also occur in individuals as young as 18 years old, with most definitions stating that onset should be between 18-65 years to be classified as '_________ onset dementia' (EOD).earlyThe general causes of dementia in younger people are similar to those in the elderly and include the following broad groups: 1. ____________ dementia: Alzheimer disease (the most common cause), frontotemporal dementia, dementia with Lewy bodies and Parkinson disease dementia 2. vascular disease: vascular dementia (usually arising from cerebral infarctions) and cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) 3. encephalopathy associated with systemic ___________ disease, e.g., SLE, Sjogren syndrome, and Behcet disease 4. non-__________ disorders, e.g., mitochondrial disease or leukodystrophies 5. others, e.g., Huntington disease or complications from alcohol abuse1. neurodegenerative 3. autoimmune 4. metabolicDiagnosis can be problematic as there may be a number of other potential causes for memory loss, behavioral or personality changes, including: 1. psychiatric disorders such as ____________ 2. psychosocial stressors 3. pathological causes, e.g. tumors or vascular malformation 4. severe _________ that can induce cognitive impairment1. depression 4. OSAAs there's no specific test available to diagnose early onset dementia, common investigations are likely to include: 1. physical and neurological exam 2. cognitive testing 3. ______ screening 4. lab tests such as blood counts or cerebrospinal fluid analysisMRIWhile there's no effective cure for dementia, medications such as cholinesterase inhibitors and N-methyl D-aspartate (NMDA) receptor agonists can be used to alleviate symptoms. 1. Cholinesterase inhibitors act by increasing cholinergic transmission and can provide some benefit to individuals with dementia. Those with a new diagnosis of ___________ disease should be given a trial of this treatment which may assist with cognitive function, although the extent of the expected effect is modest. 2. True or False: There's no definitive evidence that treatment with cholinesterase inhibitors will change the course or prognosis of the disease. 3. Memantine (Namenda) is a NMDA receptor antagonist, which acts by blocking the stimulation of NMDA receptors. It has been shown to have some beneficial effects in those with moderate or __________ disease, but the long term outcome remains uncertain.1. Alzheimer 2. True 3. severeHuntington disease is an autosomal dominant condition that causes a progressive neurodegenerative disorder that causes choreiform movements, psychiatric issues, and dementia. 1. True or False: Because HD is an autosomal dominant disorder, the abnormal gene only needs to be present in one half of a gene pair for the individual to develop the disorder. 2. _________ testing is the only way to discover which members of an at-risk family have inherited the gene before the signs of the illness manifest.1. True 2. GeneticThe presence of the gene for Huntington disease means that, at some point in the future, the first signs of the disease will begin to develop. It can begin at any time from the first to the eighth decade of life, but most present between the ages of ____ to 42 years of age.35The illness begins insidiously, usually in one of the following ways: 1. change in usual behavior (e.g., depression, moodiness, unreasonable outbursts of angers) 2. unusual jerky, fidgety movements of the ______ body and limbs, and face (i.e., chorea) 3. unsteadiness of the hands or feet, causing _____ and a tendency to be clumsy 4. an increasing inability to sustain certain simple voluntary acts, a condition called ___________2. upper 3. falls 4. impersistenceOver the years, HD progresses at a variable rate. 1. The ungainly, jerky movements increase, causing frequent falls and making walking difficult. 2. Speech usually becomes slurred and there's difficulty in __________. 3. Some sufferers become confused, forgetful, angry, unreasonable, have hallucinations, and can at times become violent. Axial ____ is used to identify caudate atrophy seen in HD and to rule out other causes of symptoms.2. swallowing 3. MRIThe prognosis of the disease is not favorable. 1. There's an increased risk of _________ associated with the psychiatric symptoms common in HD. 2. The illness usually lasts between 15-20 years, following a progressive course. Symptoms gradually become more pronounced, and cognitive decline is _________. 3. Treatment can relieve some of the symptoms, for example, anti-depressants (SSRIs), anti-psychotics such as haloperidol (Haldol) and chlorpromazine (Thorazine), mood stabilizers (lithium), and botulinum toxin (Botox) for dystonia or jaw clenching, but at present there's no known ______.1. suicide 2. inevitable 3. cure___________ refers to a group of symptoms that resemble those seen in Parkinson disease (PD) but are secondary to another disease or disorder.Parkinsonism1. In general, the causes of Parkinsonism are split into two major groups: primary and __________. 2. Additionally, there are the Parkinson _____ syndromes that have symptoms similar to those seen in idiopathic Parkinson disease, but these disorders have a complex presentation that reflect the involvement of multiple neurological systems. 3. In general, is the the prognosis of those with Parkinson plus syndromes better or worse than those who have idiopathic PD?1. secondary 2. plus 3. worseClassification of Major Parkinsonian Syndromes (info on this notecard is information only): Primary Parkinsonism: Parkinson disease - sporadic and familial Secondary Parkinsonism: Drug-induced, dopamine agonists and depletors Hemiatrophy Hydrocephalus - normal pressure hydrocephalus Hypoxia Infectious - post-encephalitic Metabolic Toxin Trauma Tumor Vascular; multi-infarct state Parkinson-Plus Syndromes: Cortical-basal ganglionic degeneration Dementia syndromes - Alzheimer disease - Diffuse Lewy body disease - Frontotemporal dementia Multiple system atrophy syndromes - Shy-Drager syndrome - Motor Neuron disease - parkinsonism Progressive supranuclear palsyPD is one of the more common neurological disorders in older individuals. It's the second most common neurodegenerative disorder in the US after Alzheimer disease. The prevalence is expected to rise as the baby boomers age. It's a chronic, progressive neurodegenerative disorder characterized by: 1. ________ at rest, which improves with movement, and may initially be unilateral 2. muscle stiffness or rigidity (often described as cogwell rigidity, which is muscle tension that gives way in little jerks when the muscle is passively stretched) 3. ___________ (a slowness in executing movement) 4. postural instability, loss of balance1. tremor 3. bradykinesia1. Other motor symptoms of PD include gait disturbances, autonomic dysfunction, loss of _________ expression, micrographia (small handwriting), dysphagia, muffled speech, and visual changes. 2. Non-motor symptoms include cognitive dysfunction and dementia, psychosis, altered sleep patterns, ________ disorders, and sensory disturbances.1. facial 2. moodPD is caused by a deficiency of a neurotransmitter called dopamine, usually as a result of the degeneration of dopamine-producing cells in the substantia nigra, a part of the ______ ganglia.basal1. Dopaminergic neurons occur in pathways essential for motor performance and influence the initiation, planning, and execution of __________. 2. In PD, the lack of __________ results in increased inhibitory activity within the motor loop of the basal ganglia, resulting in the movement-related symptoms typical of the disorder. 3. True or False: By the time clinically overt symptoms are apparent, 60-80% of dopaminergic neurons have been lost.1. movements 2. dopamine 3. True1. Diagnosis of PD is by clinical exam with confirmation by MRI, brain US, and _____ scans. 2. The prognosis of PD is usually one of slowly progressive disability and restriction in __________, but some individuals do have a more aggressive form of the disease that's associated with a higher mortality.1. PET 2. activities1. Daily _________ helps keep those with PD active longer. 2. ___________ can be a serious negative feature affecting someone living with a chronic disease. 3. Increased mortality is associated with the severity of extrapyramidal symptoms and the presence of _________. 4. Other causes of mortality are related to choking and aspiration, falls and infection. Is it true that most will die with PD rather than PD being the cause of death?1. exercise 2. Depression 3. dementia 4. Yes1. ___________ is the principal treatment of PD and can reduce symptoms in idiopathic PD.Levodopa (L-dopa)1. L-dopa is a natural precursor to dopamine and is able to cross the blood-brain barrier. Once inside the brain, it converts into __________ and relieves some of the symptoms of the disease. 2. The drawback of using L-dopa is the existence of significant side effects (nausea, postural hypotension, confusion, hallucinations, and involuntary movements), so initiation of treatment is delayed until symptoms become __________.1. dopamine 2. disabling1. Long-term treatment with levodopa has its limitations. The drug produces extra dopamine stores in the brain. However, as the disease follows its natural course, the capacity to store extra dopamine _________. 2. The benefit produced by each dose is gradually reduced, and patients experience breakthrough symptoms known as motor ____________. True or False: Levodopa has not been shown to reduce morbidity and mortality in PD.1. diminishes 2. fluctuations 3. FalseLevodopa is not the only drug treatment available. Other drugs used for PD are: 1. levodopa combined with carbidopa (Sinemet), which prevents the conversion of dopamine in the liver before it reaches the ________ 2. catechol-O-methyl transferase inhibitors tolcapone (Tasmar) and entacapone (Comtan), which extend the effectiveness of levodopa 3. dopamine agonists: apomorphine (Apokyn), bromocriptine (Parlodel), pramipexole (Mirapex), and ropinirole (Requip), which stimulate dopamine ________ and don't require conversion in the brain 4. the selective MAO-B (monoamine oxidase B) inhibitor selegiline (Eldepryl): It's believed to be neuroprotective and helps in the treatment of PD by restricting the action of MAO-B, which metabolizes the dopamine; it's most effective when used ________ in the disease and later as an adjunct to L-dopa 5. Stalevo is a combination of Sinemet and entacapone and is used for later stages of the disease when motor fluctuations occur1. brain 3. receptors 4. early1. Because dopamine and acetylcholine exist in an electrochemical balance in the brain, and PD disrupts this balance, anticholinergic drugs trihexyphenidyl (Artane) and benztropine (Cogentin) are used to improve rigidity and _______. 2. Stress reduction is necessary, as stress increases __________.1. tremors 2. symptomsIn recent years, it's become apparent that long-term treatment with L-dopa is not the perfect treatment for PD. After 5 years of treatment with levodopa, patients find that the medication wears off earlier with breakthrough __________. Improvement in surgical techniques has led to a resurgence of interest in possible surgical intervention.symptomsSurgical techniques currently used to treat PD include lesioning, deep brain _________, and implantation of fetal tissue.stimulationLesioning involves selective damage (i.e., causing a lesion) to certain cells within specific areas of the brain. 1. An electrode is inserted and once the tip reaches the target site, a lesion is created by passing an electric current through the electrode. The main target sites are in the ________ (thalamotomy) and the globus pallidus (pallidotomy). 2. Lesioning is used to treat drug-resistant _______ and may also have a benefit on rigidity, but it doesn't improve bradykinesia or akinesia, which is __________.1. thalamus 2. tremor, irreversibleDeep brain stimulation involves implantation of an electrode into specific target sites in the brain. 1. The wire is connected to an implantable pulse generator (IPG) under the skin in the chest. On a day-to-day basis, the stimulation can be switched on and off by the patient using a small magnet. It's used to treat all of the main ___________ of PD. 2. Unlike lesioning, this procedure is __________.1. symptoms 2. reversibleFor several years, research has been carried out into the possibility of replacing the dead and dying dopamine-producing cells with transplanted brain tissue from human fetuses. The hope is that fetal substantia nigra tissue will produce _________ and correct the problems concerned with dopamine deficiency. Results so far have been very mixed and the treatment is considered experimental.dopamineWhen underwriting PD, the following features need to be considered: 1. symptoms since time of diagnosis: stable or progressive 2. _______: localized (one limb) or widespread (arms, legs, and/or head) 3. dementia, if present 4. treatment: response to treatment, adverse reactions to treatment 5. extent of ________, if any 6. quality of life: effect on avocation, vocation, ADLs, psychological adjustment to illness, available support systems2. tremor 5. disabilityThe term muscular __________ refers to a group of genetically-determined disorders characterized by progressive muscle weakness (myopathy) and atrophy of skeletal muscle.dystrophyThe more common genetic types of muscular dystrophy include: 1. X-linked: Duchenne, Becker, and Emery-Dreifuss muscular dystrophies (affects 50% of males whose mother is a carrier of the defect) 2. autosomal _________: limb-girdle 2A, 2B, 2C, congenital Emery-Dreifuss, and distal dystrophies (both parents transmit the defect) 3. autosomal __________ (infant inherits the chromosome from either parent carrying the defect): facioscapulohumeral, limb-girdle 1A, 1B, 1C, etc, myotonic type 1 and 2, Emery-Dreifuss, and distal muscular dystrophies2. recessive 3. dominantCharacteristic of muscular dystrophies are electrical signals that are transmitted by nerves, with a corresponding failure of _________ to respond.musclesTesting is based on the type of muscular dystrophy suspected and includes: 1. _________ testing 2. creatine phosphokinase (CPK): parallels the degree of muscle necrosis 3. electromyography 4. muscle _________ 5. EKG, echo1. genetic 4. biopsy1. True or False: The clinical severity of muscular dystrophy varies widely and can show variation within a single genetic type. 2. As a group, muscular dystrophies rank among the most frequent of ___________ diseases.1. True 2. inheritedDuchenne and Becker forms of muscular dystrophy are rarely seen at the time of underwriting as they're associated with limited life expectancy. But it's important to note that a small percentage (5-10%) of female carriers show some degree of muscle weakness and can develop dilated ____________, with or without apparent muscle weakness.cardiomyopathyEmery-Dreifuss muscular dystrophy (EDMD), also known as humeroperoneal dystrophy, affects both males and females and is characterized by a trio of manifestations: 1. __________ occur within the first decade of life before there's any clinical evidence of muscle weakness, usually affecting the Achilles tendon, elbows, and posterior cervical muscles. 2. By 10-20 years of age, there's a slowly progressive muscle weakness, and wasting occurs proximally in the upper limbs, distally in the lower limbs and, at times, affects facial muscles. True or False: Significant disability is rare before adulthood. 3. ___________ is common, usually presenting as cardiac conduction defects ranging from sinus bradycardia and prolongation of the PR interval to complete heart block.1. Contractures 2. True 3. Cardiomyopathy1. Facioscapulohumeral muscular dystrophy (FSHMD) is named for the muscle groups that are usually affected first: __________ muscles and shoulder girdle. 2. It's an autosomal _________ inherited disorder with an infantile and classic variety.1. facial 2. dominant1. Infantile FSHMD is early onset, rapidly progressive, with marked weakness of ________ muscles. 2. It affects shoulder and hip girdles, causing lordosis (forward tilt at the hips) and hyperextension of the head and knees. Confinement to a wheelchair is usually required before age ____. 3. This form is associated with epilepsy, mental retardation, and profound _______ loss.1. facial 2. 10 3. hearingIn classic FSHMD, onset is in the second and third decades. 1. Facial muscle involvement is early. Muscles of the upper arm and shoulder are involved, with atrophy of the biceps and triceps. "Scapular ___________" is a cardinal feature. 2. Foot extensor and pelvic girdle muscles become involved later in the disease. Cardiac ________ and conduction defects have been noted. Retinal vascular disease and deafness can also be complications.1. winging 2. arrhythmias1. With FSHMD, DNA testing, to identify the mutation, and muscle ___________ are used to confirm a diagnosis. 2. Physical _________ and surgical fixation of the scapulae to the posterior thorax can improve function.1. biopsy 2. therapyTo date, at least 15 genetically different types of limb girdle muscular dystrophy (LGMD) have been identified. Biopsy with immunofluorescence reveals a reduction in one of several muscle ________.proteinsAutosomal ______ forms of LGMD are rare and, in general, are less severe than autosomal _______ forms.dominant, recessiveRecessive forms of LGMD have onset of symptoms between ages 6-18 that are progressive and have varying degrees of: 1. proximal muscle weakness and atrophy, particularly in the ____ and shoulders 2. involvement of calf muscles 3. incidence of ____________ 4. presence of elevated creatine kinase1. hips 3. cardiomyopathy1. The earliest symptoms of LGMD are waddling gait, _____ walking, exercise intolerance, and painful muscle swelling. 2. Cognitive function is usually _______. 3. Treatment is aimed to increase function and correct deformities.1. toe 2. normalMyotonic muscular dystrophy has two genetic forms, types 1 and 2. 1. Onset is in the teens and early ________. 2. Symptoms are variable and affect multiple organ systems. Muscle weakness of the face, arms, and legs is present. 3. _________ (inability of the muscle to relax quickly after voluntary contraction) is often present. 4. Non-neurological findings include _________ problems, fainting, dizziness, constipation, cataracts, and glandular dysfunction.1. twenties 3. Myotonia 4. heartWith myotonic muscular dystrophy, because the prognosis varies widely among forms of muscular dystrophy, knowing the specific _________ is important in determining the underwriting assessment.diagnosisInformation obtained when underwriting muscular dystrophies should include: 1. the precise _________ 2. results of all investigations, to include specialists' reports where available 3. confirmation regarding any cardiac involvement 4. the rate of progression 5. current level of _________1. diagnosis 5. disabilityMotor neuron disease (MND) is a progressive degeneration of motor neurons in the spinal cord, brainstem, and _______. The precise clinical picture depends on the extent of motor neurons affected by the disease process.cortex1. Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease, with the risk increasing after the age of ____. 2. True or False: It involves a degeneration of both the upper and lower motor neurons in the brain and spinal cord. 3. Signs and symptoms include weakness, spasticity, hyperreflexia, muscle ________, and gait disorders. 4. Degeneration of frontal and cortical neurons in the brain is often noted as well.1. 60 2. True 3. atrophyALS is a clinical diagnosis based on history and physical findings, supported by electromyography, motor nerve conduction studies, and single fiber nerve stimulation studies, as well as by the exclusion of other diagnoses. ______ is part of all evaluations of motor neuron disease.MRI1. As ALS progresses (usually over 2-5 years), there is progressive weakness and loss of __________ muscle function. 2. Muscles controlling _________ action are usually unaffected, and sensory symptoms are essentially absent. 3. Mild cognitive impairment, emotional lability, and bulbar signs (abnormal jaw reflexes, dysarthria, dysphagia, and drooling) can be present. 4. Weakness of the __________ and respiratory muscles is common in later stages, and ventilatory support is often necessary.1. voluntary 2. sphincter 4. diaphragm1. Riluzole (Rilutek) is used to slow the progress of ALS and delay the need for __________ support. 2. The NewRx Diaphragm Pacing System helps certain patients who have ALS-related breathing issues until there's a need for full ventilator assistance. Death is associated with progressive __________ failure.1. ventilatory 2. respiratoryProgressive bulbar palsy is a variant of ALS. 1. It involves slowly progressive weakness and wasting of muscles innervated by the _________ motor nuclei. 2. Dysarthria, difficulty chewing, and _________ are common. Upper extremity weakness can be present. 3. Mortality is associated with choking and aspiration __________.1. brainstem 2. dysphagia 3. pneumoniaPrimary lateral __________ is a rare, slowly progressive disease in which primarily upper motor neurons are affected but mild lower motor neuron symptoms can be present.sclerosisSigns of symptoms of primary lateral sclerosis mainly affect the arms and legs with: 1. stiffness and spasticity (involuntary muscle movements) 2. muscle weakness, without ________, of the arms and legs 3. dysarthria secondary to involvement of ________ muscles2. atrophy 3. facial1. At onset, symptoms of primary lateral sclerosis can be _________. 2. Balance becomes a problem as the disease progresses and the risk of _____ increases. 3. Late in the course, non-invasive ventilatory support can be needed. Physical and occupational therapy help to maintain mobility and ADLs. 4. Baclofen (Lioresal), tizanidine (Zanaflex), and clonzepam (Klonopin) are used to treat spasms. 5. True or False: Though there's often progressive disability, the survival rate is good.1. asymmetrical 2. falls 5. TrueThe term spinal muscular atrophy (SMA) refers to syndromes characterized solely by lower motor neuron signs. 1. It's an autosomal ________ hereditary disorder, though there are some sporadic mutations. 2. The pathology involves loss of anterior _____ cells in the spinal cord and cranial nerve nuclei. 3. On physical exam, proximal muscle weakness, hypotonia, hyporeflexia, and muscle ________ are common findings. 4. The extent of symptoms is dependent on the type of SMA, with most experiencing clinical onset in ___________.1. dominant 2. horn 3. atrophy 4. childhood1. __________ of SMA is made using standard tests for motor neuron disease. 2. Creatine kinase is elevated in SMA type ___. Biopsy and genetic testing may be necessary.1. Diagnosis 2. 1There are four main types of SMA: type I, type II, type III, and type IV. 1. SMA type I is acute SMA, in which the _______ muscles (those that control speech, chewing, and swallowing) are often affected, which makes feeding and swallowing extremely difficult. 2. _________ is often labored due to reduced strength of the chest muscles. 3. Because of increasing overall weakness or repeated respiratory infections, the prognosis is poor and death, in the majority of children, occurs before ____ years of age.1. bulbar 2. Breathing 3. twoSMA type II is chronic SMA with an onset between 6-18 months after birth. 2. True or False: It's the least common of the SMAs. 2. Cardinal features are ____________ delays, tremor, and musculoskeletal deformities. 3. Some children can learn to walk with the aid of bracing and can survive into adulthood. However others, due to weakened chest and respiratory muscles, can become increasingly weak with respiratory infections such as pneumonia. 4. There are many cases in which the initial progressive weakness stabilizes for a period, followed by further ___________. 5. Age of death can vary greatly, from as early as 3 years of age to _________.1. False. It's the most common 2. developmental 4. deterioration 5. adulthoodSMA type III is the mildest form of SMA and onset is after 18 months of age. 1. The prognosis is generally favorable and ___________ will be possible, though there can be difficulty with some motor skills, like climbing steps. 2. Affected individuals are often functional for years before assistance is necessary, and many will have a _______ life expectancy.1. walking 2. normalSMA type IV is adult-onset SMA, characterized by insidious onset, usually in the third decade. 1. There is very slow progression of symptoms. Muscles become weak and wasted. Most commonly affected are muscles in the hips, thighs, and __________. 2. True or False: Many individuals with adult-onset SMA will have normal life expectancy.1. shoulders 2. True1. As with muscular dystrophy, because there are many types of motor neuron disease, it's essential to ascertain the precise _________ before assessment can take place. 2. This is particularly true since the prognosis for most types is ______.1. diagnosis 2. poorInformation obtained at the time of underwriting for motor neuron diseases should include: 1. precise __________ 2. results of all investigations, to include specialists' reports where available 3. rate of __________ 4. degree of disability1. diagnosis 3. progression1. Myasthenia gravis (MG) is a disorder of neuromuscular transmission characterized by muscle fatigue and weakness that increases with ___________ use. 2. Common presenting symptoms include ocular, _______ symptoms, and weakness of the muscles of the face, limbs, and respiratory system. 3. Ocular myasthenia presents as double vision (diplopia), weakness of extraocular muscles, and ptosis (drooping) of the eyelid. True or False: Over 50% of individuals presenting with ocular myasthenia ultimately develop the generalized form of the disease. 4. Generalized MG manifests with varying degrees of skeletal muscle weakness, bulbar signs such as difficulties with speech (dysarthria) and swallowing (dysphagia), fatigue with chewing, and _________ involvement. 5. For most, symptoms progress for a time, usually peaking within ____ years.1. repetitive 2. bulbar 3. True 4. respiratory 5. 3MG affects people of both sexes equally, at any age, but is more common in ________ under age of 40 and _____ over the age of 60.females, males1. MG is an _________ disease caused by the production of antibodies against the acetylcholine receptor (AChR) present at neuromuscular junctions. 2. True or False: AChR antibodies can be detected in the serum of most with generalized MG. 3. Muscle-specific receptor tyrosine kinase (MuSK) antibodies are found frequently in those with generalized myasthenia who are AChR __________. MuSK antibodies are used to clarify the clinical diagnosis.1. autoimmune 2. True 3. negative1. Those with ocular myasthenia only can be seronegative for AChR and MuSK antibodies but can be positive for anti-striated muscle antibodies that are often associated with ________. 2. The thymus gland is considered a significant factor in the pathogenesis of MG, and approx 75% of those with myasthenia will have thymic pathology, either thymoma or primary thymic __________.1. thymoma 2. carcinoma1. Myasthenic _______ usually involves generalized weakness, bulbar signs, and respiratory insufficiency. 2. A crisis can be spontaneous or triggered by _______ such as surgery, infection, or changes in medication. 3. Respiratory function is monitored by measuring _____. 4. Hospitalization with assisted ___________ is usually required for myasthenic crisis.1. crisis 2. stressors 3. forced vital capacity (FVC) 4. ventilationA complete history and neurological evaluation are necessary for MG, and serologic tests (AChR, MuSK, and anti-striated muscle antibodies) are used as confirmatory tests. Other testing includes: 1. ice pack test: an ice pack placed over the eye decreases ptosis 2. edrophonium chloride (Tensilon) test: when injected, Tensilon prevents breakdown of acetylcholine at the neuromuscular junction and produces immediate improvement in muscle __________ 3. repetitive nerve stimulation study: amplitude of stimulated muscle decreases as electrical stimulus is repeated 4. single-fiber electromyography: a specialized test that measures neuromuscular junction function. True or False: It's positive in 95% of patients with MG. 5. MRI or CT of the chest to look for _________2. strength 4. True 5. thymomaMG can be effectively managed and 30-50% of cases actually go into remission with adequate treatment. Treatment modalities include: 1. anti-cholinesterase agents: pyridostigmine (Mestinon), neostigmine 2. __________: Glucocorticoids, azathioprine (Imuran), cyclosporine, or mycophenolate mofetil (CellCept) are often required over time or if symptoms progress 3. intravenous immunoglobulins (IVIG) and plasmapheresis: used to treat myasthenic crisis, severe weakness, and elderly patients with MG 4. ________: often significantly decreases symptoms and is done in most diagnosed under age 60.2. immunotherapy 4. thymectomy1. The most common disease course of MG is that of a relapsing and __________ illness, with variable severity from mild muscle weakness to severe respiratory crisis. 2. True or False: In general, the prognosis is promising, with many having a significant improvement with treatment.1. remitting 2. TrueFactors influencing the prognosis in MG are: 1. age of onset. Is under age 60 better or worse? 2. ocular only (Myasthenia Gravis Foundation of America Clinical Classification grade 1 or II, which is more favorable) vs generalized symptoms 3. stability of the disease, remission of symptoms 4. effectiveness of treatment 5. any complications (e.g., respiratory involvement, myasthenic crises) 6. presence or absence of ________ or thymic carcinoma1. better 6. thymoma1. The spinocerebellar degenerations are a group of disorders with progressive _______, spastic paraplegia, peripheral neuropathy, blindness, rigidity, and dementia in varying combinations. 2. Most types are _________, and the gene locus has been identified for many of these.1. ataxia 2. hereditarySporadic cases of progressive ataxia, in which only one family member is affected, are a relatively common occurrence. 1. Sporadic cases need to be differentiated from those that have acquired, non-genetic causes including alcoholism, vitamin deficiencies, anti-convulsant therapy, MS, vascular disease, and primary or metastatic tumors. The prognosis of these sporadic ataxias will be depending on the underlying _______. 2. If no cause is found, then the prognosis is likely to be _____, with no specific treatment available. 3. Often there is progressive disability and reduced life expectancy.1. cause 2. poorThe hereditary ataxias comprise a heterogeneous group of disorders that are categorized by the mode of inheritance and causative gene or chromosomal locus. 1. If genetic testing is not available, MRI and CT scans can be used to identify cerebellar ______. 2. Nerve conduction studies are usually __________.1. atrophy 2. abnormalThe hereditary ataxias have 3 common features: 1. ataxia 2. _______ abnormalities 3. pathology involving the ___________2. genetic 3. cerebellum1. Friedreich's ataxia (FRDA) is an autosomal _________ disorder characterized by slowly progressive ataxia with onset usually before age 25. 2. About two-thirds of patients have ___________. 3. DM occurs in approx 10%.1. recessive 2. cardiomyopathyFriedreich's ataxia (FRDA) is typically associated with: 1. depressed tendon reflexes 2. dysarthria 3. presence of _________ reflex (great toe flexes upward and toes fan out when the sole of the foot is firmly stroked) 4. loss of positional and ________ senses2. Babinski 4. vibrationWith FRDA, about 25% of individuals have an atypical onset, described as symptoms beginning after age 25 with tendon reflexes retained and/or progression of the disease being unusually _____.slowThe rate of progression of FRDA is variable. 1. The mean age of loss of ambulation is 25, with death occurring in the mid-30s. However, survival into the 6th and 7th decades has been documented. 2. Death is often related to ___________ and diabetes, but aspiration pneumonia due to dysphagia can also shorten life expectancy.cardiomyopathy1. In general, these autosomal ________ spinocerebellar ataxias (SCAs) are characterized by progressive cerebellar ataxia with varying degrees of bulbar dysfunction, ophthalmoparesis, pyramidal (spasticity, muscle weakness, exaggerated reflexes) and extrapyramidal (akinesia, involuntary movement, tremors) signs, optic atrophy, and dementia. 2. There are various subtypes; currently 27 have been identified (SCA9 and SCA24 remain unassigned at present), each of which corresponds to a specific gene __________.1. dominant 2. mutation1. There's no _________ for the spinocerebellar ataxias, and management is based on controlling associated impairments such as diabetes, heart failure, and impaired mobility. 2. Prognosis is variable, even within subtypes. __________ and other complications can lead to heart failure, arrhythmia, mobility problems, mental deterioration, and even death. 3. True or False: Death can occur at any time from one year to many decades after diagnosis.1. treatment 2. Cardiomyopathy 3. TrueSpinocerebellar degenerations are rarely seen in underwriting, however, the following info should be obtained if the case is seen: 1. specific diagnosis 2. current functional capacity 3. age at onset 4. any evidence of associated conditions, particularly _______ 5. any evidence of _______ involvement4. diabetes 5. cardiacTransient global amnesia (TGA) is best described as a sudden, transient loss of memory. 1. Affected individuals experience a profound loss of memory for recent events as well as difficulty in retaining _____ information. 2. Individuals usually retain personal ________ despite memory loss.1. new 2. identity1. The cause for TGA is uncertain. Symptoms usually last less than ___ hours. 2. The person with TGA will often be noted to ask the same question repeatedly (e.g., "Why am I here?"). 3. Some studies have shown a temporary interruption in _____ flow to areas in the brain that are involved in memory, including the medial temporal lobe, amygdala, and hippocampus. 4. Decreased perfusion and focal abnormalities are often found in these areas on MRI and ____ scanning.1. 24 3. blood 4. PETTGA episodes must be differentiated from TIAs, complex migraines, epileptic phenomena, venous congestion, and psychogenic disorders. 1. Those experiencing TGA will usually have a lower risk burden for ____________ (HTN, HLD, DM) than those with TIAs. 2. Those over age 50 or who have a history of __________ are more likely to experience TGA.1. atherosclerosis 2. migrainesPrognosis of TGA is dependent on the underlying cause, but if no systemic disorder is found, there are few mortality implications. Treatment is not required and it's unlikely to ______.recurEvidence to collect when underwriting TGA should include: 1. results of all investigations 2. details of all medical history 3. single or recurrent _______ 4. any residual memory loss or cognitive __________3. attacks 4. impairmentReview Question: A disease involving the degeneration of both upper and lower motor neurons is: 1. ALS 2. Duchenne muscular dystrophy 3. progressive bulbar palsy 4. SMA1. ALSReview Question: All of the following statements regarding Guillain-Barre are correct EXCEPT: 1. Total body paralysis may occur. 2. Full recovery is uncommon. 3. It's an immune-mediated condition. 4. Ventilatory support can be required.2. Full recovery is uncommon.Review Question: Which of the following statements regarding Huntington disease is/are correct? A. It's an autosomal dominant disease. B. Jerky movements and clumsiness are early signs of the disease. C. Onset of symptoms is common in the second decade of life. 1. A only 2. A and B 3. B and C 4. A, B, and C2. A and BReview Question: Favorable prognostic factors regarding a spinal cord injury include: A. normal renal function B. injury to the cervical spine only C. only one limb affected 1. A only 2. B only 3. A and C 4. B and C3. A and CThe principal treatment of Parkinson disease is: 1. surgery 2. palliative care 3. deep brain stimulation 4. levodopa4. levodopa